1/
Inspired by the guru @smlungpathguy here's my first tweetorial. What do you guys think of this submandibular salivary gland? But wait for it - I'm in a generous mood, 3 images!
2/
More from the said salivary gland. Fibrosis and inflammation. Non-specific sialadenitis? Next case? But wait, there is more to come!
3/
Now here's a circumscribed collection of inflammation (above) in dalliance with an artery. And oh, I almost forgot..there are IgG4 positive cells..lots of them.. and I'm feeling lazy..I don't wish to count😁
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I may not count but but that won't stop me from asking you a question. Is this?
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Let's step back-how does one make a diagnosis of IgG4-related disease and more specifically IgG4-related sialadenitis? The criteria are similar, for the most part, regardless of the organ involved. CRITERIA 1 Inflammation (easy!), 2. Storiform fibrosis -think cart wheel
6/
THE most characteristic feature of IgG4-related disease is obliterative phlebitis - see bigger circle. An elastic stain is awfully helpful in uncovering fully obliterated veins
7/ The diagnosis of IgG4-related disease required both histological features (at least 2) AND increased IgG4 positive cells (varies by organ) AND wait for it!) IgG4 to IgG ratio > 40% (don't you love counting!)
8/
BUYER BEWARE! This is not a diagnosis for the faint hearted. And DO NOT make this diagnosis in a clinical vacuum -particularly when working with a biopsy. These 5 pillars will spare you from entry into the doghouse
9/
This is where you could go wrong! Segment of colon with an infiltrative mass and TONS and TONS of IgG4 positive cells. Your answer on the next tweet
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Your diagnosis from 9
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SYPHILIS! And NOT IgG4-related disease!!! Infection can attract more IgG4 positive cells than IgG4-related disease. Don't tell me you fell for that trap! But no sweat - you're in very good company 😁
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And that list of diseases associated with increased number of IgG4 positive cells grows by the day! The number 1 enemy on that list is peritumoral IgG4 positive cells - you undercall a malignancy - and you're in the doghouse!
13/
And that's it! Last word of wisdom (from one who has learnt the hard way), crawl to a diagnosis of IgG4-related, don't run or jog or sprint. Whatever you're initial impression, IgG4-related disease is number 9 on your differential diagnosis!
PS Gosh this was demanding!
14/
Ooh I forget - that submandibular salivary gland - classic IgG4-related sialadenitis!
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